Advanced Ovarian Yolk Sac Tumor in Nine-Year-Old Girl: Case Report and Literature Review, 2023

Abstract

Background: Yolk sac tumor or primary endodermal tumor is a rare and aggressive malignant tumor, representing 15% to 20% of germ cell tumors of the ovaries, characterized by its high sensitivity to chemotherapy, often-present adolescents from 18 to 30 years old. Case Report: This is a 9 years old female presented with abdominal pain and distention associated with constitutional symptoms and significant weight loss, which she cannot exactly quantify. She had no family history of similar problems and denies any change in bowel habit, vaginal discharge or bleeding. Besides, she had no development of any secondary sex characteristics. On physical examination, she was not in distress with stable vital signs. Chest was clear to auscultation and abdomen was distended with slight deviation to the right side, which is hard, non-tender mobile, with irregular border about 20x18cm size, reaching to the level of umbilicus. There was no sign of fluid collection or inguinal lymph node enlargement. serum tumor markers revealed alpha fetoprotein level >1210 ng/dl, CA 125 of 70.1U/mland CT scan showed right ovarian mass with paraa ortic lymph node enlargement and attachment of the mass to the rectum. Under general anesthesia, midline incision done and about 120mla serous ascetic fluid of was evacuated. Hard, irregular right adnexa mass was identified and a huge retroperitoneal mass fixed to big blood vessels with multiple enlarged para aortic lymph nodes was identified. The omentum was with multiple kicks and the liver surface was irregular with multiple nodules at its lower edges. Peritoneal fluid cytology revealed malignant metastasis and the histopathology analysis was consistent with yolk sac tumor. She was staged surgically as 4B and discharged after clinical improvement to go abroad for chemotherapy treatment. Conclusion: The histopathology, cytology and intraoperative clinical finding were consistent with serum tumor markers that she had advanced stage yolk sac tumor with poor prognosis. Community and health professionals’ awareness for early recognition of the disease for better prognosis and initiation of pediatric chemotherapy in Eritrea is crucial, as these diseases are highly responsive to radio-chemotherapy.